Spina bifida case marks advances in fetal surgery

Hope for tiniest of patientsFor the first time in Texas, a delicate surgery in the womb has given a spina bifida patient the chance for a better life

TODD ACKERMAN, Copyright 2011, HOUSTON CHRONICLE

Published 5:30 am, Monday, August 1, 2011

Photo: Karen Warren, Houston Chronicle

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Ivan and Colette Hagler and their daughter, Faith, at Children's Memorial Hermann Hospital on July 8, four days after her birth. Faith's fetal surgery will allow her to walk, doctors say.

Ivan and Colette Hagler and their daughter, Faith, at Children's Memorial Hermann Hospital on July 8, four days after her birth. Faith's fetal surgery will allow her to walk, doctors say.

Photo: Karen Warren, Houston Chronicle

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This photo from another operation illustrates how fetal surgery is done.

This photo from another operation illustrates how fetal surgery is done.

Photo: Texas Children's Hospital

Spina bifida case marks advances in fetal surgery

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Colette and Ivan Hagler thought an ultrasound would merely reveal the sex of their baby in April when they got the bad news: Their little girl had spina bifida.

The Haglers researched the disabling birth defect on the Internet and learned the best hope might be fetal surgery, the still brave, often controversial new world where doctors remove the expectant mother's uterus and operate on the tiniest of patients. Its risks weren't lost on Colette.

"I had a lot of anxiety," said Colette, a Dallas school district employee. "I thought and thought and thought, then put my fears on the back burner. I wanted to give my daughter the best outcome possible."

Colette had the procedure at Houston's Children's Memorial Hermann Hospital in May, then in early July gave birth to Faith, 3 pounds, 9 ounces. It'll be a couple years before Faith's overall outcome is clear — the surgery is not a cure - but doctors were confident last week that she'll be able to walk instead of needing a wheelchair.

The surgery, a first in Texas for spina bifida in the womb, marks a new era. After three decades as rarely performed experimental treatment, usually when the baby was likely to die, fetal surgery has arrived as mainstream therapy for non-life-threatening conditions.

A federally funded study published earlier this year found that prenatal surgery for the most severe type of spina bifida doubled the likelihood the patient would walk unaided at 2½ and halved the chance he or she would need permanent tubes implanted after birth to remove water from the brain.

The study, 10 years in the making, is expected to not just bring fetal surgery for spina bifida to academic centers around the nation, but to stimulate greater interest in tackling other conditions before birth.

It hasn't been an easy road for fetal surgery. Its boldness attracted media attention when it was first performed in the early 1980s, but quickly triggered criticism because of the risks to the mother and a lack of proof that the benefits outweighed the costs. The early years were a time of dramatic successes and heart-wrenching failures.

Earliest interventions

The harshest criticism was levied at fetal surgery for spina bifida, the most common disabling birth defect in the United States. The hole in the spine can cause at least some leg paralysis and can also result in urinary and bowel difficulties, brain fluid and learning difficulties. About 2,000 babies a year are born with it in the U.S. Hundreds more are terminated after diagnosis, an amount doctors hope will decrease with the wider access to fetal surgery.

The condition is typically operated on one to three days after birth, but in the late 1990s a handful of centers began intervening in the womb, figuring the earlier they treated the condition, the better the results. When criticism followed, the procedure was stopped except for a clinical trial, conducted at hospitals in Nashville, San Francisco and Philadelphia, the procedure's pioneers.

It was that trial, stopped early because the evidence was so compelling that prenatal surgery was better than postnatal, that brought the Haglers to Houston this spring. At surgery, Colette was 23½ weeks pregnant, within the 19-to-26-week window to which trial participants were confined, and hopeful God would watch over her.

"It was pretty straightforward surgery," said Dr. KuoJen Tsao of the University of Texas Medical School at Houston, the pediatric surgeon for the case. "There weren't any difficulties opening the uterus, the gap was in a good place and everything went smoothly."

Tsao is nevertheless quick to acknowledge the surgery's delicate nature. Doctors use a special stapling device that cuts through the uterine wall and pinches off blood vessels to prevent bleeding, but one accidental cut to the placenta could unleash enough blood that neither fetus nor mother would survive.

She felt kicking

Doctors sealed the gap and placed Faith, less than one pound at the time, back in Colette's uterus, sewing the incision back up with the help of a microscope. Carrying the child after the surgery, Colette felt every kick, a good indication of the health of the baby's legs but hard on the healing incision. She gave birth nearly nine weeks later, earlier than doctors had hoped but in generally good health.

Texas Children's Hospital doctors, who've done open and minimally invasive fetal surgery for a number of conditions, anticipate they'll do their first for spina bifida in September. They're taking what they describe as "a calculated approach," sending doctors to the centers in the study, becoming more expert in surgery techniques before doing the first one.

They cited downsides from the study: mothers were more likely to experience ruptured membranes and deliver prematurely; babies born prematurely were more likely to have breathing problems; and all the women who had the surgery would have to give birth in the future by Caesarean section. They added that until centers become more expert at the surgery, their outcomes won't be as good as those in the study.

A dramatic procedure

Still, doctors last week were excited at the field's seeming maturation. With better technology and refined techniques, they expressed hope for new work with hydrocephalus, a brain fluid buildup for which fetal surgery in the 1980s showed no benefit; and more work with diaphragmatic hernia, a hole in the diaphragm that pushes abdominal contents into the chest cavity.

The greatest advances should come they said, when doctors are able to do more minimally invasive procedures, known as fetoscopic instead of laproscopic. Such interventions would minimize risks due to bleeding.

Until then, there's no denying open fetal surgery's dramatic nature, not just to families like the Haglers, beaming at the baby they expect to take home this week, but to doctors themselves.

"It's really the most amazing experience to look inside a womb, see unborn children whose eyes are still fused like puppies and watch them move around," said Dr. Kenneth Moise Jr., a fetal interventionist at Texas Children's. "Anybody who watches our surgeries just oohs and aahs. We're in a place no one's ever been."